The Pathology of Bovine Mannosidosis
The Pathology of Bovine Mannosidosis
复制标题
牛甘露糖苷病的病理学
DOI：
10.1177/030098587801500201
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发表时间：
1978
影响因子：
2.4
通讯作者：
K. G. Thompson
中科院分区：
文献类型：
--
作者：
Robert D. Jolly;K. G. Thompson
安格斯犊牛甘露糖苷中毒是一种与溶酶体酶α-甘露糖苷酶缺乏相关的遗传性致死性疾病。其特征为神经元、巨噬细胞、固定网状内皮细胞、外分泌上皮细胞空泡化，以及较小程度的各种其他细胞空泡化。甘露糖醇被证明是膜结合囊泡，被认为是次级溶酶体，其中储存了由糖蛋白不完全降解产生的含甘露糖的寡糖。这些糖蛋白可以通过异噬、自噬或分泌吞噬进入溶酶体系统。胰腺中存在的酶原颗粒的存储囊泡表明，分泌颗粒的不完全降解可能有助于解释其他外分泌细胞的广泛空泡化。提示这种颗粒可能通过分泌吞噬进入溶酶体系统。
Mannosidosis of Angus calves is an inherited lethal disease associated with a deficiency of the lysosomal enzyme α-mannosidase. It is characterised by vacuolation of neurons, macrophages, fixed reticuloendothelial cells, exocrine epithelial cells and to a lesser extent a wide variety of other cells. Vacuoles are shown to be membrane-bound vesicles and are considered to be secondary lysosomes in which are stored mannose-containing oligosaccharides that result from incomplete degradation of glycoproteins. Such glycoproteins may enter the lysosomal system by heterophagy, autophagy or crinophagy. The presence in the pancreas of zymogen granules in storage vesicles indicates that incomplete degradation of secretory granules might help explain the widespread vacuolation of other exocrine cells. It is suggested that such granules may enter the lysosomal system by crinophagy.